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Open access

Chronic lymphocytic leukemia/small lymphocytic lymphoma arising in the pituitary gland: illustrative case

Hang Zhou, Xiaowei Zhang, Xin Jia, Liang Jia, and Qingjiu Zhang

BACKGROUND

The authors describe a 60-year-old female who underwent a correlative examination for an accidental scalp injury, revealing a sellar mass, which was surgically excised and pathologically confirmed to be a non-Hodgkin’s small B-cell lymphoma. These findings in combination with the immunophenotype led to a final diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma. Previous studies have shown that hematological solid tumors occurring in the pituitary gland are extremely rare, and there are only approximately three other cases of living patients with similarities to this case, all of which had ambiguous expression of subsequent hematological treatment.

OBSERVATIONS

In this case, the authors used an endoscopic approach to completely excise the tumor. Follow-up of the patient was continued after surgery, and the patient is currently receiving standardized treatment with zanubrutinib.

LESSONS

This patient did not have any previous history of tumor, had a good postoperative recovery with a normal quality of life, and still receives hormone replacement and zanubrutinib on a standardized basis. This is a complete case that has not been previously reported and reveals the diagnostic and therapeutic process of rare diseases in the sellar area.

Open access

The infundibulochiasmatic angle and the favorability of an endoscopic endonasal approach in type IV craniopharyngioma: illustrative case

Guilherme Finger, Maria Jose C Ruiz, Eman H Salem, Matthew D Marquardt, Kyle C Wu, Lucas P Carlstrom, Ricardo L Carrau, Luciano M Prevedello, and Daniel M Prevedello

BACKGROUND

Lesions located in the floor of the third ventricle are among the most difficult to access in neurosurgery. The neurovascular structures can limit transcranial exposure, whereas tumor extension into the third ventricle can limit visualization and access. The midline transsphenoidal route is an alternative approach to tumor invading the third ventricle if the tumor is localized at its anterior half and a working space between the optic apparatus and the pituitary infundibulum exists. The authors introduce the “infundibulochiasmatic angle,” a valuable measurement supporting the feasibility of the translamina terminalis endoscopic endonasal approach (EEA) for resection of type IV craniopharyngiomas.

OBSERVATIONS

Due to a favorable infundibulochiasmatic angle measurement on preoperative magnetic resonance imaging (MRI), an endoscopic endonasal transsellar transtubercular approach was performed to resect a type IV craniopharyngioma. At 2-month follow-up, the patient’s neurological exam was unremarkable, with improvement in bitemporal hemianopsia. Postoperative MRI confirmed gross-total tumor resection.

LESSONS

The infundibulochiasmatic angle is a radiological tool for evaluating the feasibility of EEA when resecting tumors in the anterior half of the third ventricle. Advantages include reduced brain retraction and excellent rates of resection, with minimal postoperative risks of cerebrospinal fluid leakage and permanent pituitary dysfunction.

Open access

A sellar-suprasellar malignant optic pathway glioblastoma in the absence of prior radiation therapy: illustrative case

Mestet Yibeltal Shiferaw, Tsegazeab Laeke Teklemariam, Abenezer Tirsit Aklilu, Dejen Teke Gebrewahd, Bereket Hailu Mekuria, Ermias Fikru Yesuf, and Taye Jemberu Robele

BACKGROUND

To date, only a few cases of sellar and suprasellar glioblastomas have been reported even though high-grade glioma constitutes the most common adult brain tumor, commonly arising in the cerebral hemispheres. It arises de novo from astrocytes within the optic nerve, optic chiasm, or optic tracts and is quite challenging to diagnose and treat. To the authors’ knowledge, there are 72 cases (including this one) of optic glioma malignancies in the medical literature, 30 corresponding to glioblastomas.

OBSERVATIONS

The authors present the diagnostic considerations and challenges, management strategies, and clinical course of a very large sellar-suprasellar glioblastoma in a 19-year-female who had never received radiation therapy or prior surgery.

LESSONS

Sellar-suprasellar glioblastomas, although extremely rare, are known to occur and pose challenges in their diagnosis and preoperative treatment planning. The presence of diffusion restriction on diffusion-weighted magnetic resonance imaging in a mass lesion that has ring and nodular postcontrast enhancement in addition to absent calcification on computed tomography should be alert to the possibility of a high-grade mass. This is extremely important for preoperative patient counseling and planning for the multimodal treatments, because sellar-suprasellar glioblastomas carry a poorer prognosis than the common benign mass lesions in the region.

Open access

Occult neurohypophyseal germinoma discovered during the course of long-term diabetes insipidus: illustrative case

Hironori Yamada, Ryokichi Yagi, Akihiro Kambara, Yoshihide Katayama, Yuichiro Tsuji, Ryo Hiramatsu, Naokado Ikeda, Masahiro Kameda, Naosuke Nonoguchi, Motomasa Furuse, Shinji Kawabata, Toshihiro Takami, and Masahiko Wanibuchi

BACKGROUND

The authors report a case of occult neurohypophyseal germinoma detected in a patient with long-term diabetes insipidus. Central diabetes insipidus is the initial symptom in 95% of cases of neurohypophyseal germinoma. In occult neurohypophyseal germinomas, no abnormalities are seen on magnetic resonance imaging (MRI) at the onset of symptoms. It can take several months or even years for these changes to be detected on MRI.

OBSERVATIONS

A 20-year-old male was diagnosed with central diabetes insipidus at the age of 17 years, and gonadal and adrenal corticosteroid insufficiency was noted at the age of 19 years. Head MRI showed an enlarged and enhanced pituitary stalk. He was referred to our department for a suspected neoplastic lesion. Endoscopic transsphenoidal biopsy indicated a pure germinoma. He was treated with chemotherapy and radiotherapy and then was discharged.

LESSONS

In this case, new imaging findings appeared 19 months after the onset of diabetes insipidus, and the pathological diagnosis was made after almost 24 months. Because the patient had a history of growth hormone deficiency and had a positive test result for diabetes insipidus, occult neurohypophyseal germinoma was suspected, and periodic contrast-enhanced MRI monitoring was deemed essential.

Open access

Natural history of pituitary carcinoma with metastasis to the cervical spine: illustrative case

Nicholas T. Gamboa, Christopher Wilkerson, Bornali Kundu, Brandon A. Sherrod, Andrew T. Dailey, and William T. Couldwell

BACKGROUND

Pituitary carcinoma is a rare tumor of the adenohypophysis with noncontiguous craniospinal dissemination and/or systemic metastases. Given the rarity of this malignancy, there is limited knowledge and consensus regarding its natural history, prognosis, and optimal treatment.

OBSERVATIONS

The authors present the case of a 46-year-old woman initially treated with invasive prolactin-secreting pituitary macroadenoma who developed metastatic disease of the cervical spine 6 years later. The patient presented with acutely worsening compressive cervical myelopathy and required posterior cervical decompression, tumor resection, and instrumented arthrodesis for posterolateral fusion.

LESSONS

This case underscores the importance of long-term monitoring of hormone levels and having a high clinical suspicion for metastatic disease to the spine in patients presenting with acute myelopathy or radiculopathy in the setting of previously treated invasive secreting pituitary adenoma.

Open access

Pituitary macroadenoma causing vision loss in Wyburn-Mason syndrome: illustrative case

Nicholas F. Hug, David A. Purger, Heather E. Moss, and Robert L. Dodd

BACKGROUND

Wyburn-Mason syndrome (WMS) is a neurocutaneous disorder consisting of vascular malformations of the brain, eye, and skin. These include characteristically high-flow intracranial and intraorbital arteriovenous malformations (AVMs) that present commonly with visual deterioration, headache, and hemiplegia. Complete removal of these lesions is challenging. Most patients are followed closely, and intervention occurs only in the setting of worsening symptoms secondary to AVM growth or hemorrhage. Here the authors present the first known case of a patient with WMS and a pituitary macroadenoma.

OBSERVATIONS

A 62-year-old man with a 30-year history of WMS with right basal ganglia and orbital AVMs and right eye blindness presented for new-onset left-sided vision loss. A pituitary adenoma was identified compressing the optic chiasm and left optic nerve. Magnetic resonance imaging and digital subtraction angiography studies were obtained for surgical planning, and the patient underwent an endoscopic transnasal transsphenoidal resection, with significant postoperative vision improvement.

LESSONS

Given the variable presentation and poor characterization of this rare syndrome, patients with WMS presenting with new symptoms must undergo evaluation for growth and hemorrhage of known AVMs, as well as new lesions. Further, in patients undergoing intracranial surgery, extensive preoperative imaging and planning are crucial for safe and successful procedures.

Open access

Papillary craniopharyngioma in a patient following resection of nonfunctioning pituitary adenoma: illustrative case

David J. Park, Akash Mishra, Danielle Golub, Jian Y. Li, Karen S. Black, and Michael Schulder

BACKGROUND

Although craniopharyngioma and pituitary adenoma are common tumors of the sellar or suprasellar region, the development of papillary craniopharyngioma in the same sellar region after resection of a nonfunctioning pituitary adenoma has not been reported.

OBSERVATIONS

Here the authors report the first case of craniopharyngioma that developed long after resection of a pituitary adenoma. A 66-year-old male patient underwent endoscopic transsphenoidal resection for a large sellar mass, which histopathologically confirmed the diagnosis of a pituitary adenoma. He had an excellent recovery after surgery. For several years, he had no clinical or imaging evidence of tumor recurrence and then was lost to follow-up. Seven years after the initial surgery, the patient returned with a one-month history of visual field defects, and imaging confirmed a heterogeneous, cystic suprasellar mass. Endoscopic transsphenoidal resection of the tumor was performed, and histological examination showed it to be a papillary craniopharyngioma.

LESSONS

Neurosurgeons should be aware that after pituitary adenoma resection, a recurrent mass could be a craniopharyngioma, with implications for very different management recommendations.